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Fibrous dysplasia of the jaws: Integrating molecular pathogenesis with clinical, radiological, and histopathological features
Author(s) -
Pereira Thaís dos Santos Fontes,
Gomes Carolina Cavalieri,
Brennan Peter A,
Fonseca Felipe Paiva,
Gomez Ricardo Santiago
Publication year - 2019
Publication title -
journal of oral pathology and medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.887
H-Index - 83
eISSN - 1600-0714
pISSN - 0904-2512
DOI - 10.1111/jop.12797
Subject(s) - fibrous dysplasia , gnas complex locus , pathology , mccune–albright syndrome , craniofacial , medicine , dysplasia , pathogenesis , differential diagnosis , biology , genetics , precocious puberty , gene , psychiatry , hormone
Fibrous dysplasia is a non‐neoplastic developmental process that affects the craniofacial bones, characterized by painless enlargement as a result of bone substitution by abnormal fibrous tissue. Postzygotic somatic activating mutations in the GNAS1 gene cause fibrous dysplasia and have been extensively investigated, as well as being helpful in the differential diagnosis of the disease. Fibrous dysplasia may involve one (monostotic) or multiple bones (polyostotic), sporadically or in association with McCune‐Albright syndrome, Jeffe‐Lichenstein syndrome, or Mazabreud syndrome. This review summarizes the current knowledge on fibrous dysplasia, emphasizing the value of integrating the understanding of its molecular pathogenesis with the clinical, radiological, and histopathological features. In addition, we address important aspects related to the differential diagnosis and patient management.